Department of Urology, The Third Medical Centre, Chinese People's Liberation Army (PLA) General Hospital, Beijing, 100039, China; Medical School of Chinese People's Liberation Army (PLA), Beijing, 100039, China.
Department of Pathology, The First Medical Centre, Chinese PLA General Hospital, Beijing, 100039, China.
Hum Pathol. 2022 Nov;129:60-70. doi: 10.1016/j.humpath.2022.07.010. Epub 2022 Aug 14.
Papillary renal neoplasm with reverse polarity (PRNRP) is a newly defined entity with distinct histomorphology and recurrent KRAS mutation. In this study, we aimed to identify and analyze the clinicopathological, immunohistochemical (IHC), and molecular features of PRNRP in our center and to evaluate its differential diagnosis with other tumors with which it is easily confused: clear cell papillary renal cell carcinoma (CCPRCC), oncocytic papillary renal cell carcinoma (OPRCC), and papillary renal cell carcinoma type 1 (PRCC1). Nephrectomy specimens of PRNRP (n = 15), CCPRCC (n = 11), and OPRCC (n = 12) were retrieved from our pathology archives. We also selected typical cases of PRCC1 (n = 15) as a control group. PRNRP accounted for 3.05% (15/492) of all PRCC cases at our center. The median follow-up period was 41.3 months. All PRNRP cases were pT1N0M0, and only one involved recurrence (1 year after surgery). IHC analysis showed diffuse staining of CK7, EMA, and GATA3 but weak or negative staining of CD10, CD117, p504s, and vimentin in the PRNRP samples and distinctive IHC features in the other three tumor types. KRAS mutation was detected in 4/10 PRNRP cases. Among the 40 most commonly mutated genes identified, 5 (BCLAF1, PDE4DIP, NCOR1, PARP4, and PABPC1) have actionable alterations. Our study supports the suggestion that PRNRP is an entity distinct from CCPRCC, OPRCC, and PRCC1.
具有反向极性的乳头状肾肿瘤 (PRNRP) 是一种新定义的实体，具有独特的组织形态学和复发性 KRAS 突变。在本研究中，我们旨在确定和分析我们中心 PRNRP 的临床病理、免疫组织化学（IHC）和分子特征，并评估其与其他易混淆的肿瘤的鉴别诊断：透明细胞乳头状肾细胞癌（CCPRCC）、嗜酸细胞性乳头状肾细胞癌（OPRCC）和乳头状肾细胞癌 1 型（PRCC1）。从我们的病理档案中检索了 PRNRP（n=15）、CCPRCC（n=11）和 OPRCC（n=12）的肾切除术标本。我们还选择了典型的 PRCC1 病例（n=15）作为对照组。PRNRP 占我们中心所有 PRCC 病例的 3.05%（15/492）。中位随访时间为 41.3 个月。所有 PRNRP 病例均为 pT1N0M0，仅 1 例复发（术后 1 年）。IHC 分析显示 CK7、EMA 和 GATA3 弥漫性染色，但 PRNRP 样本中 CD10、CD117、p504s 和波形蛋白染色弱或阴性，而其他三种肿瘤类型具有独特的 IHC 特征。在 10 例 PRNRP 病例中检测到 KRAS 突变。在鉴定的 40 个最常见突变基因中，有 5 个（BCLAF1、PDE4DIP、NCOR1、PARP4 和 PABPC1）具有可操作性改变。我们的研究支持 PRNRP 是一种与 CCPRCC、OPRCC 和 PRCC1 不同的实体的观点。
